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Flashcards in Paediatric Cervical spine Deck (20):
1

What is Klippel- feil Syndrome?

Multiple abnormal segments of cervical spine

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2

What is the pathophysiology of Klippel- Feil syndrome?

  • Failure of normal formation & segementation of cervical somites at 3-8 week gestation

3

Name any associated conditions with Klippel- feil Syndrome?

  • Congential scoliosis
  • Sprengle's deformity
  • Renal disease
  • basiliar invagination
  • atantloaxial instability
  • congential heart disease

4

What are the signs and symptoms of Klippel- Feil Syndrome?

Symptoms

  • Stiff neck

Signs

  • Low posterior hair line
  • short webbed neck
  • Limited cervical ROM
  • high scapula- Sprengel's 
  • jaw anormalies
  • partial loss of hearing
  • torticolis
  • scoliosis

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5

What imaging is useful in Klippel- Feil syndrome?

Xrays

  • Ap, lateral & odontoid
  • lateral - basilar invagination
    • Atantolaxial instability- ADI >5mm
    • Degenerative changes of cervical spine
    • calcification within intervertebral space- resoluton 6/12

6

What is the tx of Klippel- feil syndrome?

non operative

  • Observation with activity modification
    • asymptomatic patients
    • most common presentation
    • councelling to avoid putting the neck at risk during certain activties

Surgical

  • Surgical decompression and fusion
    • for basilar invagination
    • chronic pain
    • myelopathy
    • assoc atlantoaxial instability
    • adj level disease if symptomatic

 

7

what is this

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  • Congential muscular torticollis ** 
  • Rotatory atantloaxial instability
  • Klippel - feil Syndrome
  • Infection
  • Tumour
  • Trauma

8

Define congential muscular torticollis?

  • A congential packaging disorder typically caused by contraction of Sternocleidomastoid

9

What is the aetiology of congential muscular torticollis?

  • True aetiology unclear
  • Some suggest intrauterine compartment syndrome of SCM
  • non SCM causes include
    • trauma
    • infection
    • tumour
    • ophthalmology
    • vestibular

10

What other disorders is congential muscular torticollis associated with?

  • other packaging disorders
  • Devlepmental dysplasis of hips - DDH = 20%
  • Metatarsus adductus
  • traumatic delivery
  • plagiocephaly- head asymmetry
  • congential altano-occiptial abnormalities

11

What is seen on examination of a child with congential muscular torticollis?

  • Head tilted - laterally to side of SCM tightness
  • Chin rotated away from side of SCM
  • Paplable neck mass & fibrosis noted within forst 4 weeks of life

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12

What are the indications for imaging? 

what imaging modality is useful?

Xrays

  • If no palpable mass if felt so that other conditions of torticollis can be ruled out
    •  rotatory atlanto-axial instabilty
    • Klippel- Feil syndrome

USS

  • If palpable mass is felt
  • can help distinguish from congential muscular torticollis for more serious neurological or osseous abnormalities

13

What is the tx of congential muscular torticollis?

Non operative

  • Passive stretch
  • conditions for <1 year
  • limitations <30 degrees
  • stretch include stretch lateral away from affected side and chin towards affected side
  • 90% respond to passive stretch

Operative

  • z plasty lengthening or distal biploar release of SCM
  • conditions > 1 year
  • limitations greater in 30o

14

What is atlantoaxial rotatory instablity?

  • C1-C2 rotatory instability caused by subluxation/facet dislocation
  • common cause of childhood torticollis
  • spectrum of disease from subluxation to full facet dislocation

15

What is the cause of atlantoaxial rotatory stability?

  • trauma
  • Retropharyngeal abscess- Grisel's disease
  • Less common
    • Down's syndrome
    • RA
      Tumours
    • congential anomalies

16

What is the pathology of atlantoaxial rotatory instability?

  • thought to be due to ligaments intact
  • transverse ligament intact
    • spinal stenosis can only happen with severe rotation & facet dislocation
  • Transverse ligament is disrupted
    • a component of atherolithesis >5mm then spinal canal stenosis can occur with less rotation- 45 degrees
    • vertebral arteries maybe at risk

17

Can you describe the classification of atlantoaxial rotatory instability?

  • Type 1- unifacet dislocation- transverse lig intact
  • Type 2- unifacet dislocation w ant displacment 3-5mm
    • transverse ligament torn
  • Type 3- bilateral anterior facet displacment >5mm
  • type 4- posterior displacement of atlas

18

What are the symptoms of atlantoaxial rotatory instability?

Symptoms

  • Tilted head
  • neck pain
  • headache

Signs

  • ispilsateral rotation and contralateral tilt of head in relation of C1
  • controlateral sternocleidmoastoid may be spastic
  • reduced cervical rotation

19

What imaging is useful in dx of atlantoaxial rotatory instability?

Xray

  • AP 
  • Odontoid peg
  • lateral
  • flexion/extension

Dynamic CT

  • gold standard
  • take ct stright forward then in max rotation to the right/left
    • you will see fixed rotation of C1/C2 which does not change with dynamic rotation

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20

What is the tx of atlantoaxial rotatory instability?

Non operative

  • Soft collar, anti-inflammatory medication, exercise programme
    • subluxation <1 week
    • many pts spontaneously reduce before seeking medical team
  • Head halter traction & bracing
    • subluxation pesistent >1 week
    • small amounts 5lbs 
    • either hospital/at home for up to 3 weeks
    • muscle relaxants/analgesics maybe required
  • Halo Traction & bracing
    • subluxation presists >1 month

Operative

  • Posterior C1-2 Fusion
    • ​if subluxation presists >3months
    • Neurological deficit present

 

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